title: [Epidermolysis bullosa acquisita: literature review].
abstract: BACKGROUND
  Epidermolysis bullosa acquisita (EBA) is the rarest of the autoimmune bullous diseases (AIBD). It is defined as an AIBD secondary to production of antibodies directed against type VII collagen and then binding to anchoring fibrils in the basal membrane zone (BMZ) of the skin and the Malpighian mucosa.
  
  
  OBJECTIVE
  To evaluate risk factors, different clinical forms and diagnostic methods, and the efficacy of treatments.
  
  
  METHODS
  The articles were identified by a search of PubMed and Embase from the initial creation of these databases through to March 2009. We selected generalised reviews and meta-analyses, cases involving unusual and/or serious clinical presentations, studies of immunological tests and homogeneous retrospective series regarding therapy.
  
  
  RESULTS
  Of the 206 articles analysed, only two were of an adequate level of proof, with four of intermediate level, and all the others of only low level. EBA affects all age groups (from newborn infants to the very elderly) with a slight predominance in female subjects. Diagnosis must be considered in subjects with black skin of African origin. A drug-induced origin of the disease was reported in 11% of cases of IgA-EBA. Classical EBA (30 to 50% of cases), resembles epidermolysis bullosa hereditaria (EBH), with fragile skin, non-inflammatory bullae, dystrophic scars and milia. Numerous atypical and misleading forms exist. Evocative signs are the presence of mucosal lesions and/or scars. The severity of EBA is determined by the extent of cutaneous lesions, and ophthalmological, ENT and/or oesophageal involvement. Crohn's disease is associated in 25% of cases of EBA. Unequivocal diagnosis is provided by direct immunoelectron microscopy (IEM). Therapeutic efficacy has been reported for dapsone, sulphapyridine and colchicine in milder forms, and for cyclosporine, mycophenolate mofetil, rituximab, intravenous immunoglobulins and extracorporeal photochemotherapy in resistant and severe forms. A number of authors have reported inefficacy of systemic corticosteroids, even in high-dose regimens, with the development of corticosteroid dependence in certain cases.
  
  
  CONCLUSIONS
  In the absence of any therapeutic trials, it is difficult to select optimal treatment; however, the benefit/risk ratio of systemic corticosteroid treatment is unfavourable.
authors.affiliation: Service de dermatologie, Centre de référence maladies rares national, pour les dermatoses bulleuses toxiques et auto-immunes, sites Avicenne et Saint-Louis, hôpital Avicenne, 125 rue de Stalingrad, Bobigny, France.
authors.forename: C
authors.initials: C
authors.lastname: Le Roux-Villet
authors.forename: C
authors.initials: C
authors.lastname: Prost-Squarcioni
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use; D001324:Autoantigens / Q000276:immunology; D002648:Child; D002675:Child, Preschool; D003078:Colchicine / Q000627:therapeutic use; D024143:Collagen Type VII / Q000276:immunology; D003131:Combined Modality Therapy; D003424:Crohn Disease / Q000150:complications; D003622:Dapsone / Q000627:therapeutic use; D016107:Epidermolysis Bullosa Acquisita / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology / Q000276:immunology / Q000628:therapy; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007159:Immunologic Tests / Q000379:methods; D007166:Immunosuppressive Agents / Q000008:administration & dosage / Q000627:therapeutic use; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008875:Middle Aged; D010778:Photochemotherapy; D016032:Randomized Controlled Trials as Topic / Q000706:statistics & numerical data; D012189:Retrospective Studies; D012307:Risk Factors; D000069283:Rituximab
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D001324:Autoantigens; D024143:Collagen Type VII; D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents; D000069283:Rituximab; D003622:Dapsone; D003078:Colchicine